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Background: Robot-assisted thoracoscopic surgery (RATS) thymectomy has been increasingly performed for treating thymic epithelial tumors in recent years. However, there are very limited reports on the long-term oncologic outcomes after RATS thymectomy, particularly in comparison to Video-assisted thoracoscopic surgery (VATS). This study aimed to compare the perioperative and long-term oncological outcomes between RATS and VATS. Methods: The study was conducted on 180 consecutive patients undergoing RATS or VATS between July 2016 and December 2019, 85 of whom underwent RATS, and 95 of whom underwent VATS. A 1:1 matched propensity score-matched analysis was performed and the perioperative and long-term oncologic outcomes of the two groups compared. Result: RATS group experienced a shorter operation time (median: 100 min vs. 120 min; P = 0.039) and less blood loss (40.00 ml vs. 50.00 ml, P = 0.011). RATS demonstrated a significantly lower conversion rate to open surgery compared to VATS, with only two patients requiring conversion in the RATS group as opposed to ten patients in the VATS group (3.03% vs. 15.15%, P = 0.030). In the RATS group, the 5-year progression-free survival rate was 87.70%, and the 5-year tumor-related survival rate was 92.31%, demonstrating no statistically significant difference compared to those in the VATS group. Conclusion: Compared with VATS, robotic thymectomy demonstrated excellent perioperative outcomes, and RATS achieved long-term oncologic outcomes comparable to those of VATS. RATS thymectomy could be considered as an effective alternative approach for treating thymic epithelial tumors. [ABSTRACT FROM AUTHOR]

Background: We present the case of a rare occurrence of an anterior mediastinal mature teratoma extending into the neck, commonly referred to as a cervicothoracic mature teratoma. Case presentation: A 19-year-old female presented with right-sided neck pain and swelling, which were found to be attributed to a 14 cm cystic lesion originating from the right thyroid lobe and extending into the mediastinum. A diagnosis of mediastinal teratoma with extension to the neck was made. Robot-assisted thymectomy was initiated but was complicated by dense tumor adherence to the superior vena cava and brachiocephalic veins, prompting a switch to a midline sternotomy. Simultaneous resection of the right thyroid lobe was performed due to inflammation. The transition to a midline sternotomy allowed successful excision of the tumor, which was confirmed to be a mature teratoma confined to the thoracic region. The patient's favorable postoperative course led to discharge on day 5 with no recurrence at nine months. Conclusions: Emphasizing the challenges and the importance of prompt intervention in the management of mediastinal teratomas with neck extension. [ABSTRACT FROM AUTHOR]

Background: Surgical approach in the treatment of thymoma is varied. This retrospective study aimed to evaluate the outcomes of different surgical approaches in the treatment of thymoma. Methods: From January 2020 to December 2023, a total of 208 patients underwent thoracoscopic surgical treatment and were diagnosed with thymoma by postoperative pathological result in our institution. We collected and then analysed potential factors including general conditions, surgical approaches, preoperative clinical symptoms, surgical procedures, postoperative hospital stay, postoperative drainge, postoperative pain score, postoperative complications and postoperative follow-up. Results: Of the 208 patients, 98 patients underwent surgery via subxiphoid approach (47.1%) and 110 patients via lateral intercostal approach (52.8%). The duration of the surgical procedure (P = 0.02), intraoperative blood loss (P = 0.045), total postoperative drainage (P = 0.021), postoperative drainage per day (P = 0.004) and Postoperative pain score (Day1 P = 0.010 and Day2 P = 0.002)showed a significant difference between these two groups. 31 patients (14.9%) received postoperative radiotherapy. One patient died due to postoperative radiotherapy. No recurrence occurred in one-year follow-up. Conclusions: The present study not only showed that subxiphoid approach was a safe and feasible technique for thymoma, with a potentially faster postoperative recovery and less postoperative pain. [ABSTRACT FROM AUTHOR]

OBJECTIVES Minimally invasive thymectomy is an accepted approach for early-stage thymic epithelial neoplasia, reducing pain and length of stay compared with open surgery. In this study, we compare robotic and video-assisted thymectomy to assess pathological resection status, overall and disease-free survival. METHODS Data were retrieved from the European Society of Thoracic Surgeons prospectively maintained thymic database. Eighty-two international centres were invited to participate in the ESTS registry. Thirty-seven centres agreed to take part. We included all patients who had undergone complete thymectomy for malignancy through a minimally invasive approach and excluded patients in whom complete data were not available. RESULTS Between October 2001 and May 2021, a total of 899 patients with thymic malignancy underwent minimal access surgical resection and were included in the study. A propensity matched analysis was conducted with interrogation of 732 patients. Median age was 55 years, and 408 (56%) patients were female. Propensity matched was performed with 1:1 matching for surgical approach (video assisted = 366, robot assisted = 366). Robot-assisted surgery conferred significantly lower odds of incomplete resection (R1; 0.203 95% CI 0.13–0.317; P  < 0.001). However, there was no difference in terms of overall and disease-free survival between the 2 techniques. CONCLUSIONS In this analysis, after adjusting for thymoma stage, the odds of incomplete surgical resection were higher in patients undergoing video-assisted surgery than robotic. However, there was no difference in overall or disease-free survival. With data maturation and increased follow-up, this would need repeat analysis and perhaps may provide more credence to the concept of a prospective randomized study to compare outcomes in thymic epithelial neoplasia by surgical approach with a standardized pathological work-up. [ABSTRACT FROM AUTHOR]

Introduction: Myasthenia gravis is an autoimmune disease that is charecterized by production of acetyl cholinesterase receptor auto antibodies. This is a retrospective study done in 45 patients who were offered trans-sternal thymectomy in such patients. Objectives: Adequate pre operative preparation with immunosuppressive agents and myasthenia gravis modifying drugs, plasmapheresis before surgery helps optimize out comes. Material and methods:Retrospectively data was collected from the years 2017-2020 in 45 patients who underwent trans-sternal thymectomy for myasthenia gravis and results analysed. Results: There has been no mortality noted in this study. 13 patients needed post operative management of myasthenic crisis. There was no reduction in the dosage of pyridostigmine. However, there has been a significant reduction in the dosage of steroids and azathriopine. Conclusion: Presence of thymoma, positive AChR Antibodies, moderate to severe airway disease are significant predictors of perioperative crisis. Prior adequate optimization before surgery is of utmost importance to prevent postoperative myasthenia crisis. Thymectomy is a very safe and effective procedure. [ABSTRACT FROM AUTHOR]

Abstract Background Surgical approach in the treatment of thymoma is varied. This retrospective study aimed to evaluate the outcomes of different surgical approaches in the treatment of thymoma. Methods From January 2020 to December 2023, a total of 208 patients underwent thoracoscopic surgical treatment and were diagnosed with thymoma by postoperative pathological result in our institution. We collected and then analysed potential factors including general conditions, surgical approaches, preoperative clinical symptoms, surgical procedures, postoperative hospital stay, postoperative drainge, postoperative pain score, postoperative complications and postoperative follow-up. Results Of the 208 patients, 98 patients underwent surgery via subxiphoid approach (47.1%) and 110 patients via lateral intercostal approach (52.8%). The duration of the surgical procedure (P = 0.02), intraoperative blood loss (P = 0.045), total postoperative drainage (P = 0.021), postoperative drainage per day (P = 0.004) and Postoperative pain score (Day1 P = 0.010 and Day2 P = 0.002)showed a significant difference between these two groups. 31 patients (14.9%) received postoperative radiotherapy. One patient died due to postoperative radiotherapy. No recurrence occurred in one-year follow-up. Conclusions The present study not only showed that subxiphoid approach was a safe and feasible technique for thymoma, with a potentially faster postoperative recovery and less postoperative pain.

The purpose of the study was to compare and analyze the levels of KREC (kappa-deleting recombination excision circle) and TREC (T cell receptor excision circle) levels, which indirectly reflect impaired maturation of T and/or B lymphocytes, in children of the medical and biological risk group and the comparison group (patients considered relatively healthy, relative to the population).The medical and biological risk groups were:1) 15 children with operated congenital heart defects with combined thymectomy and 9 without it; the average age is 5 months ±4 months and 7 months ±3 months, respectively; and2) 27 children with relatively frequent morbidity of the respiratory tract (acute respiratory viral infection more than 8 times a year) aged 1.6±1.4 months, among whom the proportion attending a preschool institution is 20 people (74%).The comparison groups were:1) 16 relatively healthy children (health group 1) aged 1.7±1.6 years, among whom the proportion of those attending a preschool institution was 13 people (81%); and2) 48 apparently healthy newborn children, whose average age was 15±12 days.Quantitative determination of TREC and KREC was carried out using a multiplex test system developed at the Institute of Chemical Biology and Fundamental Medicine of the Siberian Branch of the Russian Academy of Sciences (Novosibirsk). The average concentrations of TREC and KREC were determined in apparently healthy newborns. Children with a high incidence of infectious respiratory morbidity (more than 8 cases of acute respiratory viral infection per year) had a significantly higher risk of developing disorders of both T cell and B cell immunity, compared with the healthy population. Children who underwent total thymectomy during surgical treatment of congenital heart defects had a higher risk of developing immunodeficiency conditions, affecting predominantly T cell immunity, in comparison with the group of children operated on with preservation of the thymus gland.

Abstract Background Myasthenia gravis is an autoimmune disease with high prevalence of thymus disorders, in which, thymectomy is considered one of the therapeutic approaches in improving the patients’ clinical outcomes. Today, thoracoscopic thymectomy has received significant attention than the classic transsternal approach due to fewer complication. Therefore, this study was designed with the aim of investigating the therapeutic outcomes of thymectomy in patients with myasthenia gravis in the Afzalipour Hospital of Kerman between 2011 and 2021. Methods The current study is a descriptive analytical study on patients with myasthenia gravis who underwent surgical thymectomy within 2011–2021. Demographic and clinical characteristics of patients from the time of operation to three years of follow-up were extracted and recorded from clinical records or by phone calls. Data were analyzed using SPSS software. Results The data of 70 patients who underwent surgical thymectomy were analyzed. Thymectomy caused a significant reduction in the severity of the disease according to the Osserman classification (P = 0.001). It also significantly reduced the use of corticosteroids (P = 0.001) and IVIG (P = 0.015) compared to the time before the surgery. Sixty-two patients (88.57%) needed to take less medicine than before surgery. Left VATS was associated with less post-operative severity of the disease (P = 0.023). There were only two deaths during the follow-up period. Conclusion Overall, the findings of the present study demonstrated that thoracoscopic thymectomy is a useful surgical approach that leads to faster recovery, reducing the severity of the disease, need for medication, and complications in patients with myasthenia gravis, In comparison with the transsternal approach.

Background: Video-assisted thoracoscopic surgery (VATS) is recognized as a safe and effective treatment modality for early-stage lung cancer and anterior mediastinal masses. Recently, novel articulating instruments have been developed and introduced to endoscopic surgery. Here, we share our early experiences with VATS major pulmonary resection and thymectomy performed using ArtiSential articulating instruments. Methods: At the Seoul Metropolitan Government-Seoul National University Boramae Medical Center, 500 patients underwent VATS pulmonary resection between July 2020 and April 2023, while 43 patients underwent VATS thymectomy between January 2020 and April 2023. After exclusion, 224 patients were enrolled for VATS major pulmonary resection, and 38 were enrolled for VATS thymectomy. ArtiSential forceps were utilized in 35 of the 224 patients undergoing pulmonary resection and in 12 of the 38 individuals undergoing thymectomy. Early clinical outcomes were retrospectively analyzed. Results: No significant differences were observed in sex, age, surgical approach, operation time, histological diagnosis, or additional procedures between the patients who underwent surgery using novel articulating instruments and the group treated with conventional endoscopic instruments for both VATS major pulmonary resection and thymectomy. However, the use of the novel articulating endoscopic forceps was associated with a significantly larger number of dissected lymph nodes (p=0.028) and lower estimated blood loss (p=0.009) in VATS major pulmonary resection. Conclusion: Major pulmonary resection and thymectomy via VATS using ArtiSential forceps were found to be safe and effective, with early clinical outcomes comparable to established methods. Further research into long-term clinical outcomes and cost-effectiveness is warranted.

The diagnosis of ocular myasthenia gravis (OMG) relies primarily on clinical history, bedside tests (such as ice pack test, fatigue test, sleep test, etc.), serum antibody measurement (AChR, MuSK, and LRP4 antibody), and electrophysiological examination (RNS, SFEMG). A reasonable combination of different ancillary tests can enhance their sensitivity and specificity for diagnosing OMG. Treatment for OMG includes symptomatic treatment and immunosuppressive (IS) therapy. Patients with ophthalmoplegia typically require glucocorticoid (GC) therapy. Those who experience relapse after discontinuing GC or are intolerant to them may necessitate alternative is therapies such as traditional immunosuppressants or even targeted biologics. Early thymectomy is also an active treatment option for patients who do not respond well to symptomatic therapy or are unwilling to undergo IS therapy or have contraindications to it, or present with refractory AChR-Ab+OMG. This article reviews the recent literature progress on the diagnosis, treatment and prognosis of OMG, aiming to enhance clinicians’ understanding of OMG and encourage early diagnosis and appropriate treatment in order to achieve better outcomes. [ABSTRACT FROM AUTHOR]

Background: Myasthenia gravis is an autoimmune disease with high prevalence of thymus disorders, in which, thymectomy is considered one of the therapeutic approaches in improving the patients' clinical outcomes. Today, thoracoscopic thymectomy has received significant attention than the classic transsternal approach due to fewer complication. Therefore, this study was designed with the aim of investigating the therapeutic outcomes of thymectomy in patients with myasthenia gravis in the Afzalipour Hospital of Kerman between 2011 and 2021. Methods: The current study is a descriptive analytical study on patients with myasthenia gravis who underwent surgical thymectomy within 2011–2021. Demographic and clinical characteristics of patients from the time of operation to three years of follow-up were extracted and recorded from clinical records or by phone calls. Data were analyzed using SPSS software. Results: The data of 70 patients who underwent surgical thymectomy were analyzed. Thymectomy caused a significant reduction in the severity of the disease according to the Osserman classification (P = 0.001). It also significantly reduced the use of corticosteroids (P = 0.001) and IVIG (P = 0.015) compared to the time before the surgery. Sixty-two patients (88.57%) needed to take less medicine than before surgery. Left VATS was associated with less post-operative severity of the disease (P = 0.023). There were only two deaths during the follow-up period. Conclusion: Overall, the findings of the present study demonstrated that thoracoscopic thymectomy is a useful surgical approach that leads to faster recovery, reducing the severity of the disease, need for medication, and complications in patients with myasthenia gravis, In comparison with the transsternal approach. [ABSTRACT FROM AUTHOR]

Introduction: There are only a few studies exploring post-thymectomy outcome in patients with acetylcholine receptor antibody (AChR-Ab)-positive generalised myasthenia gravis (MG). Objective: To assess the predictors of outcome in patients with AChR-Ab-positive generalised MG who underwent thymectomy. Methods: A retrospective study of 53 patients from a single neuroscience centre in the UK. Results: The mean disease duration from diagnosis was 6.2 ± 4.3 years. Pre-thymectomy, 37 patients had mild weakness affecting muscles other than ocular muscles, 11 patients had moderate weakness and 5 patients had severe weakness. 27/53 patients had thymoma. Post-thymectomy (mean duration of 5.7 ± 4.2 years), 34 patients (64%) had a good outcome characterised by Myasthenia Gravis Foundation of America Post-Intervention Status of complete stable remission (no symptoms or signs of MG for at least 1 year without any therapy) or pharmacological remission (no symptoms or signs of MG with some form of therapy) or minimal manifestations (no symptoms of functional limitations from MG but weakness on examination of some muscles with or without some form of therapy) on last follow-up visit. Having thymomatous or non-thymomatous MG did not predict the outcome. The only variable that did predict outcome was pre-thymectomy disease severity; patients with mild weakness before thymectomy had a favourable outcome. We found an accuracy of 83% predicting outcome (95% confidence interval (CI) 60%, 100%) with a sensitivity of 84% (95% CI 68%, 94%) and specificity of 81% (95% CI 54%, 96%). Conclusion: Disease severity before thymectomy predicts outcome in patients with AChR-Ab-positive generalised MG. [ABSTRACT FROM AUTHOR]

Simple Summary: Subxiphoid single-port robotic-assisted thoracic surgery thymectomy using the single-port robotic system is currently being developed for minimally invasive surgery. This retrospective multi-institutional study is the largest study of its kind on this technique and is the first to compare the perioperative outcomes of this technique to those of subxiphoid single-port video-assisted thoracic surgery thymectomy. This novel technique was associated with a lower conversion rate to multi-port surgery, shorter chest tube drainage duration, and shorter postoperative hospital stays, demonstrating its potential as a surgical treatment option for selected patients. Subxiphoid thymectomy is a novel alternative to the transthoracic approach and sternotomy, with potential benefits, such as reduced postoperative pain and faster recovery. We previously reported the initial experience with subxiphoid single-port robotic-assisted thoracic surgery (SRATS) thymectomy using the single-port robotic system (SPS). However, the efficacy of this technique remains unknown. Thus, this study examined the multi-institutional experience with SRATS thymectomy and compared the perioperative outcomes of this technique to those of subxiphoid single-port video-assisted thoracic surgery (SVATS) thymectomy. The data of patients who underwent subxiphoid SRATS and SVATS thymectomy, performed by three thoracic surgeons at three institutions between September 2018 and May 2024, were retrospectively collected. In total, 110 patients were included, with 85 and 25 undergoing SRATS and SVATS thymectomy, respectively. After propensity score matching, 25 patients were included in each group. The SRATS group was associated with a lower conversion rate to multi-port surgery (0% vs. 20%, p = 0.05), shorter chest tube drainage duration (1.32 ± 0.75 vs. 2.00 ± 1.29 days, p = 0.003), and a shorter postoperative hospital stay (2.52 ± 1.00 vs. 5.08 ± 5.20 days, p = 0.003). Subxiphoid SRATS thymectomy using the SPS is feasible and is a good alternative to conventional thymectomy. Further studies are necessary to confirm its benefits. [ABSTRACT FROM AUTHOR]

Objective: To compare the safety and effectiveness of different surgical approaches in thymectomy: robotics, subxiphoid, lateral video‐assisted thoracoscopy surgery (LVATS) and open. Methodology: We retrospectively reviewed 68 cases of thymectomy with a robot‐assisted, subxiphoid, LVATS, open sternotomy or thoracotomy approach for thymic lesions or myasthenia gravis between July 2017 and May 2023 at a single centre. Peri‐operative outcomes (operating time, estimated blood loss, conversion rates, R0 resection, adverse events and length of stay [LOS]) were collected. Results: We observed six conversions to open (from five LVATS and one robot assisted). The median estimated blood loss was lower for LVATS (100.00 [50.0–100.0] mL) compared with open thymectomies (200.0 [150.0–400.0]; P <.001). No intra‐operative adverse events were reported in the robotics, subxiphoid or LVATS groups. In patients with thymic tumours (n = 34), R0 resection was achieved in 100% (2/2) of robotics, 83% of subxiphoid (5/6), 93% (13/14) of LVATS and 75% (n = 9/12) of open cases. The median LOS was shortest for robot assisted (1.0 [interquartile range (IQR) 1.0–3.0]), then subxiphoid (2.0 [IQR 1.0–3.0]), LVATS (2.0 [IQR 1.0–3.0]) then open (5.0 [IQR 4.0–6.0]; P <.001). Conclusions: Our results suggest that with a shorter LOS, robotics, subxiphoid and LVATS thymectomies are safe. Larger size studies are required to compare R0 resection rates between these less invasive surgical approaches. [ABSTRACT FROM AUTHOR]

OBJECTIVES The aim of this study is to demonstrate the ability of the Versius surgical system to successfully and safely complete a range of thoracic procedures aligned with Stage 2a (Development) of the Idea, Development, Exploration, Assessment and Long-term follow-up framework for surgical innovation. METHODS This prospective study included the first 30 consecutive patients who underwent robotic surgery with Versius by 2 surgeons without prior robotic experience between 1 April 2023 and 30 December 2023 [25 lung resections (wedge, segmentectomy and lobectomy) and 5 thymectomies]. There were no specific predetermined selection criteria for each case. The primary outcome was safe completion of the procedure without unplanned conversion. Secondary outcomes included intraoperative and postoperative complications, intraoperative device-related outcomes and pathology results. RESULTS Twenty-eight (93.3%) cases were completed without conversion. Both conversions were to thoracoscopy, one due to a 'console alarm' and the other due to pulmonary artery bleeding. In lung resections, median console time was 103 (90–129) min. Five (20%) patients experienced postoperative complications, most frequent was persistent air leak (16%). Median length-of-stay was 3 (2–4) days. Neither readmissions nor mortality was observed. In thymectomies, no intraoperative or postoperative complications, readmissions, reinterventions or mortality were observed. Median console time was 77 (75–89) min and median length of stay was 1 (1–1) day. CONCLUSIONS This phase 2a IDEAL-D study confirms lung resections and thymectomies are feasible with the use of Versius system, laying the foundation for larger phase 2b and 3 clinical studies within the IDEAL-D framework. [ABSTRACT FROM AUTHOR]

Introduction. The association between Sjögren's syndrome (SS) and the development of lymphoma is well known. The prevalence of lymphoma in patients with SS is 5%. Mucosaassociated lymphoid tissue (MALT) lymphoma is the most common lymphoma type in patients with SS. It is common for MALT lymphoma to develop in the stomach, while it is extremely rare in the thymus. Case report. We present a 61-year-old Caucasian male patient with primary SS, cutaneous vasculitis, and thymic MALT lymphoma. The patient had a two-year history of diffuse cutaneous palpable purpura on legs, intermittently enlarged left parotid gland, and dry mouth. The results of Schirmer's test were positive, labial salivary glands biopsy revealed a focus score = 1, serology testing showed positive anti-Ro/SS-A and anti-La/SS-B antibodies, while skin biopsy findings revealed leukocytoclastic vasculitis. Diagnosis of primary SS with extraglandular cutaneous manifestations was confirmed. Cryoglobulinemia (Cg) and monoclonal gammopathy (MG) were detected, which increased the suspicion of hematological malignancy, and additional diagnostic procedures were performed. Computed tomography of the chest revealed an enlarged, multicystically altered anterior mediastinal mass. A thymectomy was performed through video-assisted thoracic surgery. Histological findings of the tissue confirmed the presence of tumor tissue consistent with MALT lymphoma in the thymus. Induction therapy with pulse doses of glucocorticoids was applied for three days, which was continued with medium doses of the drug. The doses were gradually reduced, and hydroxychloroquine was introduced. This has shown to be an effective therapy against features of SS. Postoperative local radiotherapy was performed. Conclusion. In SS patients with CV and in the presence of Cg and MG, attention should also be paid to the eventual development of MALT lymphoma, including the rare localization in the thymus. [ABSTRACT FROM AUTHOR]

Background and purpose: This study aimed to investigate the clinical efficacy and safety of telitacicept in patients with generalized myasthenia gravis (gMG) who tested positive for acetylcholine receptor antibodies or muscle‐specific kinase antibodies and were receiving standard‐of‐care therapy. Methods: Patients meeting the eligibility criteria were randomly assigned to receive telitacicept subcutaneously once a week for 24 weeks in addition to standard‐of‐care treatment. The primary efficacy endpoint was the mean change in the quantitative myasthenia gravis (QMG) score from baseline to week 24. Secondary efficacy endpoints included mean change in QMG score from baseline to week 12 and gMG clinical absolute score from baseline to week 24. Additionally, safety, tolerability and pharmacodynamics were assessed. Results: Twenty‐nine of the 41 patients screened were randomly selected and enrolled. The mean (± standard deviation [SD]) reduction in QMG score from baseline to week 24 was 7.7 (± 5.34) and 9.6 (± 4.29) in the 160 mg and 240 mg groups, respectively. At week 12, mean reductions in QMG scores for these two groups were 5.8 (± 5.85) and 9.5 (± 5.03), respectively, indicating rapid clinical improvement. Safety analysis revealed no adverse events leading to discontinuation or mortalities. All patients showed consistent reductions in serum immunoglobulin (Ig) A, IgG and IgM levels throughout the study. Conclusion: Telitacicept demonstrated safety, good tolerability and reduced clinical severity throughout the study period. Further validation of the clinical efficacy of telitacicept in gMG will be conducted in an upcoming phase 3 clinical trial. [ABSTRACT FROM AUTHOR]

Objective. To describe the clinical features of patients with congenital heart disease (CHD) who subsequently developed systemic juvenile idiopathic arthritis (sJIA). Methods. We conducted a retrospective review of patients diagnosed with CHD and sJIA at our institution. Detailed clinical, laboratory, and radiographic data were collected from the medical record and reviewed with each patient's primary medical team. Results. Five patients with sJIA and CHD were identified. Each child had a unique cardiac anatomy, but all the patients required surgical repair during the first year of life. Four children had thymectomies at the time of cardiac surgery. Classic signs of sJIA such as fever (n = 5), rash (n = 5), and arthritis (n = 4) developed after surgical intervention in all the patients. The individuals in this cohort displayed risk factors associated with severe sJIA, including disease onset before 2 years of age (n = 5), elevated interleukin 18 levels (n = 5), baseline eosinophilia prior to initiation of biologic disease-modifying antirheumatic drugs (n = 4), and positivity for HLA-DRB1*15:01 alleles (n = 4). Macrophage activation syndrome (MAS) occurred in 3 patients and sJIA-associated lung disease (sJIA-LD) was identified in 4 patients. Two children died from complications of their cardiac and/or pulmonary disease. Conclusion. We identified an association between CHD and severe forms of sJIA. Although these findings will need to be confirmed in larger, multicenter cohorts, the results highlight the importance of considering a diagnosis of sJIA in children with CHD and remaining vigilant for complications such as MAS and sJIA-LD. [ABSTRACT FROM AUTHOR]

Background: The advent of robot-assisted thoracoscopic surgery (RATS) has completely revolutionized the modality of thymectomy, which could reportedly achieve equivalent efficacy compared with a minimally invasive approach. This study was conducted to further compare the perioperative outcomes between these two modalities. Methods: A retrospective single-center study that included patients receiving either a robotic or video-assisted thoracoscopic (VAT) thymectomy between February 2021 and January 2023 was conducted. All the patients were pathologically confirmed with thymic epithelial tumors. Clinical and pathological characteristics and perioperative outcomes were collected and compared between these two cohorts. Results: A total of 190 patients were included in this study, with 61 (32.1%) and 129 (67.9%) receiving robotic and video-assisted thymectomy, respectively. The clinicopathological characteristics were not significantly different between these 2 groups. The size of the resected specimens in the RATS cohort was larger than the VATS cohort [median (IQR), 13.0 (8.0–16.0) vs. 9.0 (6.7–12.0) cm, p < 0.001], while the procedural duration was longer for the RATS group than its counterpart [median (IQR), 105 (85–143) vs. 85 (69–115) min, p = 0.001]. Moreover, no other significant difference was observed between these two groups. Since more than half of the robotic thymectomy was performed using a subxiphoid approach, a subgroup analysis was further conducted. Similarly, the robotic group through a subxiphoid approach harbored a longer procedural duration, and the size of the specimens obtained was larger than the VATS group [median (IQR), 14.0 (11.0–16.5) vs. 12.5 (8.5–15.0) cm, p = 0.061]. Conclusions: The early clinical efficacy of robotic thymectomy was proven comparable to the established VATS approach, and such a modality might have strength when obtaining larger specimens, which could contribute to improving long-term efficacy. Despite the longer procedural duration recorded in the early stage of conducting robotic thymectomy, further accumulation would help decrease the time. [ABSTRACT FROM AUTHOR]

Purpose: Surgical patients with thymoma and myasthenia gravis (MG) must have their MG status and oncological outcomes critically monitored. We aimed to identify clinicopathological predictors of the postoperative MG status. Methods: We conducted a retrospective review of 40 consecutive surgical patients with MG-related thymomas between 2002 and 2020. The quantitative myasthenia gravis score (QMGS) and Myasthenia Gravis Foundation of America post-intervention status (MGFA-PIS) were used to evaluate postoperative MG status. Results: All patients underwent extended total thymectomy. The most common WHO type was type B2 (32%), while 65% of patients had type B1–B3 and 35% had type A–AB thymomas. Eleven patients (28%) achieved controlled MG status in MGFA-PIS 6 months after surgery. This controlled status was observed more frequently in type A–AB than in B1–B3 (57% vs. 12%, p = 0.007). In a multivariate analysis, WHO type (A–AB or B1–B3) was an independent predictor of worsening episodes of MG based on the QMGS (Type B1–B3, hazard ratio: 3.23, 95% confidence interval: 1.12–9.25). At the last follow-up, 23 patients (58%) achieved controlled MG status. The 5-year overall survival rate of all patients was 93.7%. Conclusion: The WHO type of thymoma is an informative predictor of postoperative MG status in patients with MG-related thymoma. [ABSTRACT FROM AUTHOR]

Juvenile myasthenia gravis (JMG) is a rare autoimmune disease that causes fatigable muscle weakness in children aged <18 years. There is currently no curative treatment or internationally accepted standard of care for JMG. The objective is to investigate relationships between clinical presentation, antibody status, severity of disease onset, electrodiagnostic evaluation, and response to therapy in JMG. This study was a retrospective chart review. Congenital myasthenic syndromes were excluded. Data on demographics, treatments, and outcomes were collected. Disease severity was evaluated using Myasthenia Gravis Foundation of America (MGFA) clinical classifications. We identified 84 patients with JMG at Children's Medical Center Dallas between January 2014 and February 2022. It was found that 52% of patients presented with ocular JMG (median onset age 4.5 years) and 48% with generalized JMG (median onset age 11.5 years); 81% tested positive for acetylcholine receptor antibodies. Patients were 17% non-Hispanic white, 29% Hispanic, 39% black, and 12% Asian. There was a significant difference in average MGFA scores between ethnicities (P = 0.047) and age groups (P = 0.004), with postpubertal patients having higher average MGFA scores than prepubertal patients. Seventy-one percent of patients who underwent thymectomy experienced a decrease in MGFA scores postprocedure. Our study showed that there were significant differences in disease severity between ethnicities and age groups and that most patients who underwent thymectomy showed clinical improvement. These outcomes highlight the need for additional therapies in the treatment of JMG and the importance of extending clinical trials to the pediatric population. [ABSTRACT FROM AUTHOR]

Aim: This nationwide study evaluated the clinical impact that an early thymectomy, during congenital heart defect (CHD) surgery, had on the health of children and adolescents. Methods: The subjects were patients aged 1–15 years who had undergone CHD surgery at the University Children's Hospital, Helsinki, where all CHD surgery in Finland is carried out, from 2006 to 2018. The parents or the cases and population‐based controls, matched for sex, age and hospital district, completed electronic questionnaires. We excluded those with low birth weights or a known immunodeficiency. Adjusted odds ratios (aOR) and 95% confidence intervals (CI) were calculated for prespecified outcomes. Results: We received responses relating to 260/450 (58%) cases and 1403/4500 (31%) controls and excluded 73 cases with persistent cardiac or respiratory complaints after surgery. The CHD group reported more recurrent hospitalisations due to infections (aOR 6.3, 95% CI 3.0–13) than the controls and more pneumonia episodes (aOR 3.5, 95% CI 2.1–5.6), asthma (aOR 2.5, 95% CI 1.5–4.1) and wheezing (aOR 2.1, 95% CI 1.5–2.9). Conclusion: Hospitalisation due to infections, pneumonia, wheezing and asthma was more common in children after a thymectomy due to open‐heart surgery than population‐based controls, underlining the importance of immunological follow‐ups. [ABSTRACT FROM AUTHOR]

This trial was to evaluate the efficacy of subxiphoid approach thoracoscopic thymectomy for postoperative pain control and length of hospital stay compared with a lateral intercostal approach thoracoscopic thymectomy. This multicenter, open-label, randomized clinical superiority trial enrolled 101 eligible participants clinically diagnosed with Masaoka-Koga I-II thymoma between August 15, 2021, and February 15, 2022. Each enrolled participant was randomized and underwent subxiphoid approach thoracoscopic thymectomy or lateral intercostal approach thoracoscopic thymectomy. A per-protocol analysis for each coprimary outcome was performed in addition to the main intention-to-treat analysis. In the analysis for the coprimary outcomes, the pain Visual Analog Scale score area under the curve at 0 to 7 days was lower in the subxiphoid approach thoracoscopic thymectomy group than in the lateral intercostal approach thoracoscopic thymectomy group (difference, −4.82; 98.3% CI, −8.84 to −0.80). However, there was no significant difference between the 2 groups in the length of hospital stay (difference, 0.318; 98.3% CI, −0.190 to 0.825) or cumulative opioid consumption after surgery (difference, −4.630; 98.3% CI, −9.530 to 0.272). All patients underwent complete resection, and there was no significant difference (7.84% vs 8.00%, P = 1.000) in the rate of complications between the 2 groups. No recurrence or death occurred in the postoperative 6 months. This study found improved pain and similar length of hospital stay associated with the subxiphoid approach compared with the lateral intercostal approach in patients with suspected Masaoka-Koga I-II thymoma. [Display omitted] [ABSTRACT FROM AUTHOR]

Background: Myasthenia gravis (MG) is the most common paraneoplastic disorder associated with thymic neoplasms. MG can develop after thymectomy, and this condition is referred to post-thymectomy myasthenia gravis (PTMG). Diffuse panbronchiolitis (DPB), is a rare form of bronchiolitis and is largely restricted to East Asia, has been reported in association with thymic neoplasms. Only three cases of combined MG and DPB have been reported in the literature. Case presentation: A 45-year-old Taiwanese woman presented to our hospital with productive cough, rhinorrhea, anosmia, ear fullness, shortness of breath, and weight loss. She had a history of thymoma, and she underwent thymectomy with adjuvant radiotherapy 7 years ago. Chest computed tomography scan revealed diffuse bronchitis and bronchiolitis. DPB was confirmed after video-assisted thoracoscopic surgery lung biopsy, and repeated sputum cultures grew Pseudomonas aeruginosa. She has been on long-term oral azithromycin therapy thereafter. Intravenous antipseudomonal antibiotics, inhaled amikacin, as well as oral levofloxacin were administered. Three months after DPB diagnosis, she developed ptosis, muscle weakness, and hypercapnia requiring the use of noninvasive positive pressure ventilation. MG was diagnosed based on the acetylcholine receptor antibody and repetitive stimulation test results. Her muscle weakness gradually improved after pyridostigmine and corticosteroid therapies. Oral corticosteroids could be tapered off ten months after the diagnosis of MG. She is currently maintained on azithromycin, pyridostigmine, and inhaled amikacin therapies, with intravenous antibiotics administered occasionally during hospitalizations for respiratory infections. Conclusions: To our knowledge, this might be the first case report of sequential development of DPB followed by PTMG. The coexistence of these two disorders poses a therapeutic challenge for balancing infection control for DPB and immunosuppressant therapies for MG. [ABSTRACT FROM AUTHOR]

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Abstract Myasthenia gravis (MG) is an autoimmune disease characterized by muscle fatigability due to acetylcholine receptor (AChR) autoantibodies. To better characterize juvenile MG (JMG), we analyzed 85 pre- and 132 post-pubescent JMG (with a cutoff age of 13) compared to 721 adult MG patients under 40 years old using a French database. Clinical data, anti-AChR antibody titers, thymectomy, and thymic histology were analyzed. The proportion of females was higher in each subgroup. No significant difference in the anti-AChR titers was observed. Interestingly, the proportion of AChR+ MG patients was notably lower among adult MG patients aged between 30 and 40 years, at 69.7%, compared to over 82.4% in the other subgroups. Thymic histological data were examined in patients who underwent thymectomy during the year of MG onset. Notably, in pre-JMG, the percentage of thymectomized patients was significantly lower (32.9% compared to more than 42.5% in other subgroups), and the delay to thymectomy was twice as long. We found a positive correlation between anti-AChR antibodies and germinal center grade across patient categories. Additionally, only females, particularly post-JMG patients, exhibited the highest rates of lymphofollicular hyperplasia (95% of cases) and germinal center grade. These findings reveal distinct patterns in JMG patients, particularly regarding thymic follicular hyperplasia, which appears to be exacerbated in females after puberty.

Abstract Background Cardiac herniation occurs when there is a residual pericardial defect post thoracic surgery and is recognised as a rare but fatal complication. It confers a high mortality and requires immediate surgical correction upon recognition. We present a case of cardiac herniation occurring post thymectomy and left upper lobectomy. Case presentation Initial presentation: A 48-year-old male, hypertensive smoker presented with progressive breathlessness and was found to have a left upper zone mass confirmed on CT biopsy as carcinoid of unclear origin. PET-CT revealed avidity in a left anterior mediastinal area, left upper lobe (LUL) lung mass, mediastinal lymph nodes, and a right thymic satellite nodule. Intraoperatively: Access via left thoracotomy and sternotomy. The LUL tumour involved the left thymic lobe (LTL), left superior pulmonary vein (LSPV), left phrenic nerve and intervening mediastinal fat and pericardium, which were resected en-masse. The satellite nodule in the right thymic lobe (RTL) was adjacent to the junction between the left innominate vein and superior vena cava (SVC). The pericardium was resected from the SVC to the left atrial appendage. Clinical deterioration: Initially the patient was doing well clinically on day 1, however there was sudden bradycardia, hypotension, clamminess, and oligoanuria, with raised central venous pressures and troponins. ECG: no capture in leads V1-2, but positive deflections seen on posterior leads. Echo: no acoustic windows, but good windows seen posteriorly. CXR: left mediastinal shift. Redo operation: After initial resuscitation and stabilisation on the intensive care unit, on day 2 a redo-sternotomy revealed cardiac herniation into the left thoracic cavity with the left ventricular apex pointing towards the spine, and inferior caval kinking. After reduction and repair of the pericardial defect with a fenestrated GoreTex patch, the patient recovered well with complete resolution of the ECG and CXR. Conclusion Cardiac herniation can even occur following sub-pneumonectomy lung resections and should be considered as a differential when faced with a sudden clinical deterioration, warranting early surgical correction.

Thymectomy in a number of clinical situations is an unavoidable step of cardiac surgical treatment of congenital heart defects, but the issue of its effects on development of immunity in early ontogenesis remains open. There is still debates among scientists about the risk of immune deficiency states in children who underwent thymectomy at an early age. One may see completely different opinions, both in domestic and foreign publications. A tool for resolving this issue may be provided by morpho-functional studies of the removed thymic tissue by means of modern scanning electron microscopy techniques. The objective of our study was to assess the possibilities of EMbedding and backscattered scanning electron microscopy for morphological and functional evaluation of the thymus, which was forcedly removed in the infant patients with congenital heart defects over the first weeks of life. The thymus of a newborn infant (postnatal day 27) with congenital heart disease: ventricular septal defect was examined using EMbedding and backscattered scanning electron microscopy imaging after embedding in epoxy resin. The mass of thymus gland was 15.7 g, the dimensions of thymus were: transverse, 3.4 cm; longitudinal, 4.1 cm; thickness, 1.7 cm; volume, 12.4 cm3. The study showed ability of EMbedding and backscattered scanning electron microscopy in morphological and functional assessment of thymus gland considered the central organ of the immune system. Step-bystep visualization from low to high magnifications, from tissues to cells and intracellular structures, as well as layer-by-layer examination of thymic cortex, medulla, interlobular septa and vessels, allows you to effectively assess the functionality of the thymus. This research method is sufficient for scientific research of the forcedly removed thymus since it enables us to visualize its microanatomy, allowing cell phenotyping at different layers of the thymus, studying intercellular interactions of thymocytes with reticulo-epithelial cells, subtle features of Hassall’s bodies and, finally, the process of T lymphocytes’ release from thymus gland.

Background: Thymic mucosa-associated lymphoid tissue (MALT) lymphoma is rare and is known to be associated with Sjögren's syndrome (SjS). SjS is rarely accompanied by serositis. Here, we describe the first case of postoperative cardiac tamponade and acute pleuritis in a patient with thymic MALT lymphoma associated with SjS. Case presentation: A 33-year-old woman with SjS presented with an anterior mediastinal mass on chest computed tomography, which was performed for further examination of the condition. Suspecting a thymic MALT lymphoma or thymic epithelial tumor, total thymectomy was performed. The mediastinal mass was histopathologically diagnosed as a thymic MALT lymphoma. The patient was discharged with a good postoperative course but visited the hospital 30 days after surgery for dyspnea. Cardiac tamponade was observed and drainage was performed. Four days after pericardial drainage, chest radiography revealed massive left pleural effusion, and thoracic drainage was performed. The patient was diagnosed with serositis associated with SjS and treated with methylprednisolone, which relieved cardiac tamponade and pleuritis. Conclusions: Surgical invasion of thymic MALT lymphomas associated with SjS may cause serositis. Postoperative follow-up should be conducted, considering the possibility of cardiac tamponade or acute pleuritis due to serositis as postoperative complications. [ABSTRACT FROM AUTHOR]

Myasthenia gravis (MG) is an autoimmune disease characterized by muscle fatigability due to acetylcholine receptor (AChR) autoantibodies. To better characterize juvenile MG (JMG), we analyzed 85 pre- and 132 post-pubescent JMG (with a cutoff age of 13) compared to 721 adult MG patients under 40 years old using a French database. Clinical data, anti-AChR antibody titers, thymectomy, and thymic histology were analyzed. The proportion of females was higher in each subgroup. No significant difference in the anti-AChR titers was observed. Interestingly, the proportion of AChR+ MG patients was notably lower among adult MG patients aged between 30 and 40 years, at 69.7%, compared to over 82.4% in the other subgroups. Thymic histological data were examined in patients who underwent thymectomy during the year of MG onset. Notably, in pre-JMG, the percentage of thymectomized patients was significantly lower (32.9% compared to more than 42.5% in other subgroups), and the delay to thymectomy was twice as long. We found a positive correlation between anti-AChR antibodies and germinal center grade across patient categories. Additionally, only females, particularly post-JMG patients, exhibited the highest rates of lymphofollicular hyperplasia (95% of cases) and germinal center grade. These findings reveal distinct patterns in JMG patients, particularly regarding thymic follicular hyperplasia, which appears to be exacerbated in females after puberty. [ABSTRACT FROM AUTHOR]

Background and purpose: Preoperative risk categorization of thymoma is useful for treatment decisions but remains challenging. This study focused on training radiomics models using contrast-enhanced computed tomography (CECT) images for thymoma risk categorization and validating the model's performance, reliability and generalizability in a relatively large cohort. Methods: This retrospective cohort study analyzed the clinical data of thymoma patients (Masaoka Koga I-III) who underwent thymectomy surgery at the Affiliated Chest Hospital of Shanghai Jiao Tong University School of Medicine from January 2008 to December 2017. The cohort was divided into a training group (80%) and a test group (20%) using stratified random selection. The gold standard for histologic types was based on surgically resected specimens. Low-risk histologic types included A, AB and B1. High-risk histologic types included B2 and B3. Radiomics features were extracted from manually segmented regions of interest on preoperative CECT images. Interobserver correlation and least absolute shrinkage and selection operator (LASSO) regression were used for feature selection. Model performance metrics included area under the curve (AUC) of receiver operating characteristic (ROC) curve, sensitivity and specificity. Clinical characteristics were added to the combined model. Results: A total of 478 patients (mean age 51.3±12.3 years, 48.1% was male) were included. The AUC of the clinical model, the CECT-based model, and the model using both clinical and CECT features on the test set were 0.666, 0.831 and 0.850, respectively. The best performing model had a sensitivity of 0.829 and a specificity of 0.764. Conclusion: CECT-based radiomics models showed good performance in risk categorization of thymomas. [ABSTRACT FROM AUTHOR]

Background: Current practice patterns suggest open rather than minimally invasive (MIS) approaches for thymomas >4 cm. We hypothesized there would be similar perioperative outcomes and overall survival between open and MIS approaches for large (>4 cm) thymoma resection. Methods: The National Cancer Database was queried for patients who underwent thymectomy from 2010 to 2020. Surgical approach was characterized as either open or MIS. The primary outcome was overall survival and secondary outcomes were margin status, and length of stay (LOS). Differences between approach cohorts were compared after a 1:1 propensity match. Results: Among 4121 thymectomies, 2474 (60%) were open and 1647 (40%) were MIS. Patients undergoing MIS were older, had fewer comorbidities, and had smaller tumors (median; 4.6 vs 6 cm, P <.001). In the unmatched cohort, MIS and open had similar 90-day mortality (1.1% vs 1.8%, P =.158) and rate of positive margin (25.1% vs 27.9%, P =.109). MIS thymectomy was associated with shorter LOS (2 (1-4) vs 4 (3-6) days, P <.001). Propensity matching reduced the bias between the groups. In this cohort, overall survival was similar between the groups by log-rank test (P =.462) and multivariate cox hazard analysis (HR.882, P =.472). Multivariable regression showed shorter LOS with MIS approach (Coef −1.139, P <.001), and similar odds of positive margin (OR 1.130, P =.150). Discussion: MIS has equivalent oncologic benefit to open resection for large thymomas, but is associated with shorter LOS. When clinically appropriate, MIS thymectomy may be considered a safe alternative to open resection for large thymomas. [ABSTRACT FROM AUTHOR]

Recommendations for the treatment of myasthenia gravis (MG) have been difficult to develop because of limited evidence from large randomized controlled trials. New drugs and treatment approaches have recently been shown to be effective in phase 3 studies in seropositive generalized (g) MG. One such drug is efgartigimod, a human-Fc-fragment of IgG1, with a high affinity for the endosomal FcRn. We conducted a multicenter study to evaluate the real-world clinical and safety effects of efgartigimod in 22 gMG patients. We evaluated the strategies for the timing of re-treatment with it. The participants received a total of 59 efgartigimod -treatment cycles. The median number of cycles was 2 (range 1–6). Twenty patients (86.3%) improved by at least 2 MG-ADL points after the first treatment cycle. The median MG-ADL score at baseline was 6.5 (range: 3–17) and 2.5 (range: 0–9) post-treatment (p < 0.001). A consistent improvement of at least 2 points in the MG-ADL score after each cycle occurs in 18 patients. The effect duration of the treatment was usually between 4 and 12 weeks. Two major clinical patterns of treatment response were found. Treatment with efgartigimod was also associated with significant reductions of prednisone doses Overall, the treatment was safe and associated with only minor adverse events. The single fatality was apparently due tosevere respiratory failure. We found that efgartigimod is clinically effective, may be used as a steroid sparing agent and is generally safe for gMG patients. We recommend a personalized preventive treatment approach until clinical stabilization, followed by discontinuation and periodic evaluations. [ABSTRACT FROM AUTHOR]

Kabuki Syndrome (KS) is a multisystemic genetic disorder. A portion of patients has immunological manifestations characterized by increased susceptibility to infections and autoimmunity. Aiming to describe the clinical and laboratory immunological aspects of KS, we conducted a retrospective multicenter observational study on patients with KS treated in centers affiliated to the Italian Primary Immunodeficiency Network. Thirty-nine patients were enrolled, with a median age at evaluation of 10 years (range: 3 m–21y). All individuals had organ malformations of variable severity. Congenital heart defect (CHD) was present in 19/39 patients (49%) and required surgical correction in 9/39 (23%), with associated thymectomy in 7/39 (18%). Autoimmune cytopenia occurred in 6/39 patients (15%) and was significantly correlated with thymectomy (p < 0.002), but not CHD. Individuals with cytopenia treated with mycophenolate as long-term immunomodulatory treatment (n = 4) showed complete response. Increased susceptibility to infections was observed in 22/32 patients (69%). IgG, IgA, and IgM were low in 13/29 (45%), 13/30 (43%) and 4/29 (14%) patients, respectively. Immunoglobulin substitution was required in three patients. Lymphocyte subsets were normal in all patients except for reduced naïve T-cells in 3/15 patients (20%) and reduced memory switched B-cells in 3/17 patients (18%). Elevated CD3 + TCRαβ + CD4-CD8-T-cells were present in 5/17 individuals (23%) and were correlated with hematological and overall autoimmunity (p < 0.05). In conclusion, immunological manifestations of KS in our cohort include susceptibility to infections, antibody deficiency, and autoimmunity. Autoimmune cytopenia is correlated with thymectomy and elevated CD3 + TCRαβ + CD4-CD8-T-cells, and benefits from treatment with mycophenolate. [ABSTRACT FROM AUTHOR]

• Short-term symptom changes after TE are associated with postoperative recurrence in MG. • Postoperative recurrence patients had a higher QMGS at 1 month after the operation. • rr-QMGS＜36.7% was a positive predictor for postoperative recurrence. To investigate the relationship between short-term changes in quantitative myasthenia gravis score (QMGS) after thymectomy and postoperative recurrence in myasthenia gravis (MG) patients without thymoma. A retrospective observational cohort study. The QMGS of 44 patients with non-thymomatous MG were evaluated before and 1 month after thymectomy, and the frequency and time of postoperative recurrence were recorded. The reduction rate of QMGS (rr-QMGS) was defined as (QMGS one week before thymectomy − QMGS one month after thymectomy)/ QMGS one week before thymectomy × 100 %, as an indicator of short-term symptom change after thymectomy. The receiver operating characteristic (ROC) curve was established to determine an appropriate cut-off value of rr-QMGS for distinguishing postoperative recurrence. Multivariate Cox regression analysis was applied to predict postoperative recurrence. Postoperative recurrence occurred in 21 patients (30 times in total) during follow-up. The mean annual recurrence rate was 3.98 times/year preoperatively and 0.30 times/year postoperatively. ROC analysis determined the cut-off value of rr-QMGS was 36.7 % (sensitivity 90.5 %, specificity 52.2 %). Multivariate Cox regression analysis showed that rr-QMGS＜36.7 % (hazard rate[HR]6.251, P = 0.014) is positive predictor of postoperative recurrence. Kaplan-Meier analysis showed that postoperative recurrence time was earlier in the low rr-QMGS group than in the high rr-QMGS group (12.62 vs. 36.60 months, p = 0.005). Low rr-QMGS is associated with early postoperative recurrence. Rr-QMGS can be used to predict postoperative recurrence of non-thymomatous MG. [ABSTRACT FROM AUTHOR]

Background: Thymectomy is an option for the treatment of myasthenia gravis (MG). While the open technique was most frequently performed in the past, nowadays the endoscopic approach has gained wide acceptance. Here we assessed our early experience in thoracoscopic thymectomy (TT). Methods: This case series was retrospectively conducted at Al-Thawra Modern General Hospital and included all patients diagnosed with MG who underwent TT from January 2018 to January 2024. Results: Our case series consisted of 13 predominantly female patients (61.5%), with a median age of 39.5 years. Surgeries typically lasted 50 ± 10.41 minutes, with the majority performed using a left-sided thoracoscopic approach (n=10, 77%). Immediate extubation was achieved in 10 patients (76%). Four patients experienced early postoperative complications (31%), including dyspnoea, prolonged intubation, chest infection, confusion, tracheostomy, and re-tracheostomy. There were 2 recorded deaths due to complications. Eleven patients were followed up for an average of 16.5 months, revealing that 54.5% achieved complete stable remission, 18% showed improvement with reduced symptoms and medications, and 27% remained unchanged. Conclusion: Thoracoscopic thymectomy is feasible and effective procedure for the management of MG in Yemeni patients. The observed remission and improvement rates are promising and align with global experiences. It is recommended that with proper resources and expertise, similar minimally invasive surgical approaches can be implemented in resource-limited regions. Plain Language Summary: This study was conducted to evaluate the use of thoracoscopic thymectomy (TT) for the treatment of myasthenia gravis (MG) at Al-Thawra Modern General Hospital. In this study, we included 13 predominantly female patients with a median age of 39.5 years. The most common symptoms experienced by the patients were generalized weakness and ptosis (drooping of the eyelids). During the surgeries, which typically lasted around 50 minutes, we primarily used a left-sided thoracoscopic approach. We were successful in immediately removing the breathing tube in 10 out of 13 patients. However, four patients experienced early postoperative complications, including difficulties in breathing, longer intubation, chest infection, confusion, and the need for tracheostomy. Out of the 13 patients, two unfortunately passed away due to complications. We followed up with 11 patients for an average of 16.5 months and observed that 54.5% of them achieved complete stable remission, meaning they had no MG symptoms and did not require any treatment for at least one year. Additionally, 18% of the patients showed improvement with reduced symptoms and medication usage, while 27% remained unchanged. Based on our findings, we conclude that thoracoscopic thymectomy is a safe and effective procedure for managing MG in Yemeni patients. The remission and improvement rates we observed align with experiences from around the world. We believe that with appropriate resources and expertise, similar minimally invasive surgical approaches can be implemented in resource-limited regions. [ABSTRACT FROM AUTHOR]

Patients with neuromuscular diseases are particularly vulnerable in the perioperative period to the development of pulmonary and cardiac complications, or medication side effects. These risks could include hypoventilation, aspiration pneumonia, exacerbation of underlying cardiomyopathy, arrhythmias, adrenal insufficiency, prolonged neuromuscular blockade, issues related to thermoregulation, rhabdomyolysis, malignant hyperthermia, or prolonged mechanical ventilation. Interventions at each of the perioperative stages can be implemented to mitigate these risks. A careful pre-operative evaluation may help identify risk factors so that appropriate interventions are initiated, including cardiology consultation, pulmonary function tests, initiation of noninvasive ventilation, or implementation of preventive measures. Important intraoperative issues include positioning, airway and anesthetic management, and adequate ventilation. The postoperative period may require correction of electrolyte abnormalities, control of secretions with medications, manual or mechanical cough assistance, avoiding the risk of reintubation, judicious pain control, and appropriate medication management. The aim of this review is to increase awareness of the particular surgical challenges in this vulnerable population, and guide the clinician on the various evaluations and interventions that may result in a favorable surgical outcome. [ABSTRACT FROM AUTHOR]

Background: Subxiphoid video-assisted thoracoscopic surgery (VATS) is considered a safe and feasible operation for anterior mediastinal mass resection. However, diaphragmatic injury, presented as tearing or puncturing, may occur during subxiphoid VATS despite of low incidence. This study aims to explore risk factors for diaphragmatic injury in subxiphoid VATS, as well as strategies to reduce occurrence of the injury. Methods: We retrospectively reviewed clinical records of 44 consecutive adult patients who underwent subxiphoid VATS. These patients were divided into two groups: diaphragmatic injury group and non-injury group. Perioperative outcomes and anatomic features derived from 3D CT reconstructions were compared between the two groups. Results: Significant differences were observed in operation time (223.25 ± 92.57 vs. 136.28 ± 53.05, P = 0.006), xiphoid length (6.47 ± 0.85 vs. 4.79 ± 1.04, P = 0.001) and length of the xiphoid below the attachment point on the diaphragm (24.86 ± 12.02 vs. 14.61 ± 9.25, P = 0.029). Odds ratio for the length of the xiphoid below the attachment point on the diaphragm was 1.09 (1.001–1.186), P = 0.048 by binary logistic regression analysis. Conclusions: We identified the length of the xiphoid below the attachment point on the diaphragm as an independent risk factor for diaphragm injury during subxiphoid VATS. Prior to subxiphoid VATS, a 3D chest CT reconstruction is recommended to assess the patients' anatomic variations within the xiphoid process. For patients with longer xiphoid process, a higher incision at the middle and upper part of the xiphoid process, and partial xiphoid process resection or xiphoidectomy is preferred. [ABSTRACT FROM AUTHOR]

OBJECTIVES Robotic thymectomy has been suggested and considered technically feasible for thymic tumours. However, because of small-sample series and the lack of data on long-term results, controversies still exist on surgical and oncological results with this approach. We performed a large national multicentre study sought to evaluate the early and long-term outcomes after robot-assisted thoracoscopic thymectomy in thymic epithelial tumours. METHODS All patients with thymic epithelial tumours operated through a robotic thoracoscopic approach between 2002 and 2022 from 15 Italian centres were enrolled. Demographic characteristics, clinical, intraoperative, postoperative, pathological and follow-up data were retrospectively collected and reviewed. RESULTS There were 669 patients (307 men and 362 women), 312 (46.6%) of whom had associated myasthenia gravis. Complete thymectomy was performed in 657 (98%) cases and in 57 (8.5%) patients resection of other structures was necessary, with a R0 resection in all but 9 patients (98.6%). Twenty-three patients (3.4%) needed open conversion, but no perioperative mortality occurred. Fifty-one patients (7.7%) had postoperative complications. The median diameter of tumour resected was 4 cm (interquartile range 3–5.5 cm), and Masaoka stage was stage I in 39.8% of patients, stage II in 56.1%, stage III in 3.5% and stage IV in 0.6%. Thymoma was observed in 90.2% of patients while thymic carcinoma occurred in 2.8% of cases. At the end of the follow-up, only 2 patients died for tumour-related causes. Five- and ten-year recurrence rates were 7.4% and 8.3%, respectively. CONCLUSIONS Through the largest collection of robotic thymectomy for thymic epithelial tumours we demonstrated that robot-enhanced thoracoscopic thymectomy is a technically sound and safe procedure with a low complication rate and optimal oncological outcomes. [ABSTRACT FROM AUTHOR]

Thymomas are rare tumors originating from thymic tissue and rarely metastasize. They can be diagnosed either incidentally or symptomatically when compressing or invading nearby structure. A 36-year-old man presented with significant high-grade fever, chest pain that worsens upon lying down, and dyspnea. A chest X-Ray and computed tomography followed by biopsy confirmed the diagnosis of thymoma. The management included chemotherapy cycles, followed by surgery. Pericardiectomy was performed with en-bloc thymectomy and partial resection of the infiltrating lung. Venous drainage was restored by 8/16 mm inverted bifurcated brachiocephalic-superior vena cava Dacron bypass. The pericardium was reconstructed by a synthetic Dacron patch, and the right diaphragm metastasis was resected. Neoadjuvant chemotherapy was initiated. After 3 months of follow-up, no recurrence was evidenced by computed tomography. [ABSTRACT FROM AUTHOR]

Background: Cardiac herniation occurs when there is a residual pericardial defect post thoracic surgery and is recognised as a rare but fatal complication. It confers a high mortality and requires immediate surgical correction upon recognition. We present a case of cardiac herniation occurring post thymectomy and left upper lobectomy. Case presentation: Initial presentation: A 48-year-old male, hypertensive smoker presented with progressive breathlessness and was found to have a left upper zone mass confirmed on CT biopsy as carcinoid of unclear origin. PET-CT revealed avidity in a left anterior mediastinal area, left upper lobe (LUL) lung mass, mediastinal lymph nodes, and a right thymic satellite nodule. Intraoperatively: Access via left thoracotomy and sternotomy. The LUL tumour involved the left thymic lobe (LTL), left superior pulmonary vein (LSPV), left phrenic nerve and intervening mediastinal fat and pericardium, which were resected en-masse. The satellite nodule in the right thymic lobe (RTL) was adjacent to the junction between the left innominate vein and superior vena cava (SVC). The pericardium was resected from the SVC to the left atrial appendage. Clinical deterioration: Initially the patient was doing well clinically on day 1, however there was sudden bradycardia, hypotension, clamminess, and oligoanuria, with raised central venous pressures and troponins. ECG: no capture in leads V1-2, but positive deflections seen on posterior leads. Echo: no acoustic windows, but good windows seen posteriorly. CXR: left mediastinal shift. Redo operation: After initial resuscitation and stabilisation on the intensive care unit, on day 2 a redo-sternotomy revealed cardiac herniation into the left thoracic cavity with the left ventricular apex pointing towards the spine, and inferior caval kinking. After reduction and repair of the pericardial defect with a fenestrated GoreTex patch, the patient recovered well with complete resolution of the ECG and CXR. Conclusion: Cardiac herniation can even occur following sub-pneumonectomy lung resections and should be considered as a differential when faced with a sudden clinical deterioration, warranting early surgical correction. [ABSTRACT FROM AUTHOR]

Background: There are limited data on the real-world healthcare resource use (HCRU) and management costs of myasthenia gravis (MG) in England. Objective: This study aims to assess the burden of disease for patients with MG in England. Design: A retrospective, observational cohort study of adult patients diagnosed with MG, using data from the Hospital Episode Statistics data warehouse. Methods: Patients with a first-ever recorded diagnosis of MG between 30 June 2015 and 30 June 2020 were followed up until 30 June 2021 or death, whichever occurred first. Post-diagnosis patient characteristics, treatment patterns, HCRU, and costs were described. Costs were evaluated using National Health Service reference costs. Results: A total of 9087 patients with a median follow-up time of 2.9 years (range, 1.7–4.3 years) were included. The mean age at diagnosis was 66.5 years and 53% of the patients were male. A large proportion of patients (72.8%) were admitted as inpatients during follow-up with a mean number of 1.3 admissions. Patients hospitalized for MG-related complications spent a mean of 9.7 days per patient-year in the hospital. During follow-up, 599 (6.6% of the total cohort) and 163 (1.8%) patients had a record of rescue therapy with intravenous immunoglobulin (IVIg) and plasma exchange (PLEX), respectively. Rituximab was administered to 81 (0.9%) patients and 268 (2.9%) patients underwent thymectomy. In those patients receiving rescue therapy or rituximab, >10% received at least three cycles of the same treatment. The average annual cost of hospital admissions across all patients treated with IVIg, PLEX, and rituximab were £907,072, £689,979, and £146,726, respectively. Conclusion: A majority of MG patients required hospitalization or accident and emergency attendance, resulting in high HCRU and costs. A subset of patients required rescue therapy (including IVIg and PLEX), rituximab administration, ventilation, or thymectomy. [ABSTRACT FROM AUTHOR]

Introduction: thymomas are rare tumors, they are considered the most frequent of the anterior mediastinum and originate in the thymic gland. Patient information: 55-year-old female patient, with a history of digestive disorders, occasional numbness and weakness in the upper and lower extremities, passive smoker since childhood. She suffered from COVID-19 Delta in 2021, received treatment and has no repercussions. She attended the Pulmonology Clinic due to right scapular pain and, at times, cough with whitish morning expectorations. She denied dyspnea and was afebrile. Through laboratory and imaging studies, a clinical suspicion of thymoma was reached, a diagnosis that was confirmed by the biopsy report of the lesion removed by conventional transsternal thymectomy. Conclusions: an early and timely diagnosis of thymoma must be made to guarantee greater survival for patients, for which knowledge of its imaging characteristics is of special importance. [ABSTRACT FROM AUTHOR]

Introduction: Robotic and thoracoscopic surgery are being increasingly adopted as minimally invasive alternatives to open sternotomy for complete thymectomy. The superior maneuverability range and three-dimensional magnified vision are potential ergonomical advantages of robotic surgery. To compare the ergonomic characteristics of robotic versus thoracoscopic thymectomy, a previously developed scoring system based on impartial findings was employed. The relationship between ergonomic scores and perioperative endpoints was also analyzed. Methods: Perioperative data of patients undergoing robotic or thoracoscopic complete thymectomy between January 2014 and December 2022 at three institutions were retrospectively retrieved. Surgical procedures were divided into four standardized surgical steps: lower-horns, upper-horns, thymic veins and peri-thymic fat dissection. Three ergonomic domains including maneuverability, exposure and instrumentation were scored as excellent(score-3), satisfactory(score-2) and unsatisfactory(score-1) by three independent reviewers. Propensity score matching (2:1) was performed, including anterior mediastinal tumors only. The primary endpoint was the total maneuverability score. Secondary endpoints included the other ergonomic domain scores, intraoperative adverse events, conversion to sternotomy, operative time, post-operative complications and residual disease. Results: A total of 68 robotic and 34 thoracoscopic thymectomies were included after propensity score matching. The robotic group had a higher total maneuverability score (p = 0.039), particularly in the peri-thymic fat dissection (p = 0.003) and peri-thymic fat exposure score (p = 0.027). Moreover, the robotic group had lower intraoperative adverse events (p = 0.02). No differences were found in residual disease. Conclusions: Robotic thymectomy has shown better ergonomic maneuverability compared to thoracoscopy, leading to fewer intraoperative adverse events and comparable early oncological results. [ABSTRACT FROM AUTHOR]

Abstract Background Thymoma and myasthenia gravis (MG) interact with each other. This study aimed to evaluate the effects of thymoma on neurological outcome of MG patients after thymectomy using the propensity score matching (PSM) method. Methods Consecutive patients with MG who underwent thymectomy at Beijing Hospital between January 2012 and August 2021 were retrospectively enrolled. Clinical and follow-up data were collected. Statistical analysis was performed using SPSS 23.0 software. PSM was performed to eliminate selection bias. Results A total of 456 patients were included in this study. Thymoma was present in 138 (30.3%) patients. The median follow-up time was 72 (range, 12–135) months. At the last follow-up, a lower proportion of thymomatous MG patients achieved complete stable remission (CSR) compared with non-thymomatous MG patients (P = 0.011), and the effective rate [CSR + pharmatologic remission (PR) + minimal manifestations (MM)] of thymomatous MG patients was also lower (P = 0.037). Considering time to CSR, Kaplan–Meier analysis showed thymomatous MG patients had lower cumulative CSR rate than non-thymomatous MG patients (log-rank, P = 0.019). After PSM, 105 pairs of patients were matched successfully. For the matched patients, thymomatous MG patients had a lower CSR rate and a lower effective rate (P = 0.002, 0.039, respectively), and K–M analysis still showed thymomatous MG patients had lower cumulative CSR rate (log-rank, P = 0.048). Multivariate Cox analysis demonstrated that thymoma (HR: 0.592, 95% CI 0.389–0.900, P = 0.014), older age at the time of surgery (HR: 0.971, 95% CI 0.953–0.990, P = 0.003), and preoperative course of MG > 12 months (HR: 0.474, 95% CI 0.317–0.708, P = 0.000) were negative predictive factors for CSR. Conclusions Thymoma had a negative effect on the neurological outcome of MG after thymectomy. MG patients with old age and a preoperative course of longer than one year had a lower probability of achieving CSR.

Thoracic duct ligation is a demanding procedure when the chyle leak and/or the duct itself are not identified. This report describes a new procedure using thoracoscopic closure of the chyle leak by application of a fibrin sealant patch. This strategy was successfully applied for closing (i) a small fistula due to a postoperative lesion of the lymphatic tributary vessels in one case, and (ii) a large fistula due to idiopathic rupture of the thoracic duct in another case. [ABSTRACT FROM AUTHOR]